Apotemnophilia or body integrity identity disorder (BIID) denotes a syndrome in which a person is preoccupied with the desire to amputate a healthy limb. In this report, we review the available case reports in the literature in order to enhance psychiatrists' and physicians' comprehension of this disorder. A search for the case reports available via MEDLINE was done since the first case report published by Money et al in 1977 till May 2011, using the following terms: apotemnophilia, self-demand amputation, body integrity identity disorder, and BIID. In all, 14 case reports were found relevant to our search. The desire to amputate one's healthy limb seems to be related to a major disturbance in the person's perception of one's own identity, where limb amputation can relieve temporarily the patient's feeling of distress without necessarily and uniformly adjusting the patient's own identity misperception. More investigations are needed in this domain in order to develop noninvasive treatment strategies that approach this aspect of the patient's distress within a globalist perspective. In addition, the health professionals' awareness regarding this disorder is required to ensure professional management of patients' suffering.